Phosphoribosylpyrophosphate in Man: Biochemical and Clinical Significance

Abstract

The intracellular concentration of phosphoribosylpyrophosphate (PRPP), a high-energy 1,5-substituted ribose sugar, has been demonstrated to have a critical role in the regulation of purine metabolism in man. Increased intracellular levels of PRPP may be important in the pathogenesis of excessive uric acid production observed in some patients with primary gout, the Lesch-Nyhan syndrome, and glycogen storage disease type I. In addition, the alteration of intracellular PRPP by several compounds including allopurinol, orotic acid, adenine, 2,6-diaminopurine, methylene blue, and certain trophic hormones may account for some of the effects of these agents.